Evidence map›Paper›PMID 25087226›Full record

ReviewThe Journal of allergy and clinical immunology2014

The CARD11-BCL10-MALT1 (CBM) signalosome complex: Stepping into the limelight of human primary immunodeficiency.

Stuart E Turvey, Anne Durandy, Alain Fischer, Shan-Yu Fung, Raif S Geha, Andreas Gewies, Thomas Giese, Johann Greil, Bärbel Keller, Margaret L McKinnon and 6 more

Open access · bronzeAbstract readReview
In one paragraph

Review in The Journal of allergy and clinical immunology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 84 papers.

0numbers the graph read from it
0cells of the map it votes in
84citing papers in PubMed
5.1field-weighted citation impact, top 4% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

84 citing papers in PubMed, 147 citations in OpenAlex.

  1. Review
  2. A novel linker region truncating variant in BCL10 underlies a leaky immunodeficiency phenotype.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2026
    Article
  3. Review
  4. Article
  5. Article
  6. Article
  7. Article
  8. Article
  9. Article
  10. Understanding and Managing Hyper IgE Syndromes.ImmunoTargets and therapy · 2025
    Review
  11. Review
  12. Article
  13. Transcriptome Profiling Associated withInternational journal of molecular sciences · 2024
    Article
  14. Article
  15. Article
  16. Article
  17. Review
  18. TCR Signals Controlling Adaptive Immunity against Toxoplasma and Cancer.Advances in experimental medicine and biology · 2024
    Article
  19. Review
  20. Article

24 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

16 authors at 12 institutions in 5 countries.

Stuart E TurveyDepartment of Pediatrics, Child & Family Research Institute, and BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada. Electronic address: sturvey@cw.bc.ca.
Anne DurandyNational Institute of Health and Medical Research and the Department of Immunology and Hematology, Assistance Publique-Hopitaux de Paris, Necker Children's Hospital, Paris, and Descartes-Sorbonne Paris Cité University of Paris, Imagine Institute, Paris, France.
Alain FischerNational Institute of Health and Medical Research and the Department of Immunology and Hematology, Assistance Publique-Hopitaux de Paris, Necker Children's Hospital, Paris, and Descartes-Sorbonne Paris Cité University of Paris, Imagine Institute, Paris, France; Unité d'immuno-hématologie pédiatrique, Hôpital Necker-Enfant Malades, Assistance Publique des Hôpitaux de Paris (APHP), Paris, France.
Shan-Yu FungDepartment of Pediatrics, Child & Family Research Institute, and BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Raif S GehaDivision of Immunology, Boston Children's Hospital and Department of Pediatrics, Harvard Medical School, Boston, Mass.
Andreas GewiesGerman Cancer Consortium (DKTK), partner site Munich at the Institut für Klinische Chemie und Pathobiochemie, Klinikum rechts der Isar, Technische Universität München, Munich, and German Cancer Research Center (DKFZ), Heidelberg, Germany.
Thomas GieseInstitute for Immunology, University of Heidelberg, Heidelberg, Germany.
Johann GreilDepartment of Pediatric Oncology, Hematology and Immunology, University of Heidelberg, Heidelberg, Germany.
Bärbel KellerCentre for Chronic Immunodeficiency (CCI), University Medical Center Freiburg and University of Freiburg, Freiburg, Germany.
Margaret L McKinnonDepartment of Medical Genetics, Child & Family Research Institute and BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Bénédicte NevenUnité d'immuno-hématologie pédiatrique, Hôpital Necker-Enfant Malades, Assistance Publique des Hôpitaux de Paris (APHP), Paris, France.
Jacob RozmusDepartment of Pediatrics, Child & Family Research Institute, and BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Jürgen RulandInstitut für Klinische Chemie und Pathobiochemie, Klinikum rechts der Isar, Technische Universität München, Munich, Germany.
Andrew L SnowDepartment of Pharmacology, Uniformed Services University of the Health Sciences, Bethesda, Md.
Polina StepenskyPediatric Hematology-Oncology and Bone Marrow Transplantation, Hadassah Hebrew University Medical Center, Jerusalem, Israel.
Klaus WarnatzCentre for Chronic Immunodeficiency (CCI), University Medical Center Freiburg and University of Freiburg, Freiburg, Germany.
BC Children's Hospital · CAChild and Family Research Institute · CAHeidelberg University · DEKlinikum rechts der Isar · DEAssistance Publique – Hôpitaux de Paris · FRHadassah Medical Center · ILHarvard University · USHôpital Necker-Enfants Malades · FRSorbonne Paris Cité · FRUniformed Services University of the Health Sciences · USUniversity Medical Center Freiburg · DEUniversity of Freiburg · DE

Funding

Role of DOCK8 in B Cell FunctionR01AI100315 · NIAID · BOSTON CHILDREN'S HOSPITAL · PI GEHA, RAIF SALIM · 2013 to 2017
$2.2M
B Cell immunodeficiency in BENTA diseaseR21AI109187 · NIAID · HENRY M. JACKSON FDN FOR THE ADV MIL/MED · PI SNOW, ANDREW L · 2014 to 2015
$412k
Combined SNP analysis and whole genome sequencing to discover immunodeficiency geR03AI094017 · NIAID · BOSTON CHILDREN'S HOSPITAL · PI GEHA, RAIF SALIM · 2011 to 2012
$174k
British Heart Foundation MOP133691European Research Council 322865NIAID NIH HHS AI094017NIAID NIH HHS AI100315NIAID NIH HHS R01 AI100315NIAID NIH HHS R03 AI094017NIAID NIH HHS R21 AI109187
6 · The paper itself

Abstract

Next-generation DNA sequencing has accelerated the genetic characterization of many human primary immunodeficiency diseases (PIDs). These discoveries can be lifesaving for the affected patients and also provide a unique opportunity to study the effect of specific genes on human immune function. In the past 18 months, a number of independent groups have begun to define novel PIDs caused by defects in the caspase recruitment domain family, member 11 (CARD11)-B-cell chronic lymphocytic leukemia/lymphoma 10 (BCL10)-mucosa-associated lymphoid tissue lymphoma translocation gene 1 (MALT1 [CBM]) signalosome complex. The CBM complex forms an essential molecular link between the triggering of cell-surface antigen receptors and nuclear factor κB activation. Germline mutations affecting the CBM complex are now recognized as the cause of novel combined immunodeficiency phenotypes, which all share abnormal nuclear factor κB activation and dysregulated B-cell development as defining features. For this "Current perspectives" article, we have engaged experts in both basic biology and clinical immunology to capture the worldwide experience in recognizing and managing patients with PIDs caused by CBM complex mutations.

Indexed as

Adaptor Proteins, Signal TransducingB-Cell CLL-Lymphoma 10 ProteinB-LymphocytesCARD Signaling Adaptor ProteinsCaspasesGene Expression RegulationGerm-Line MutationGuanylate CyclaseHigh-Throughput Nucleotide SequencingHumansImmunologic Deficiency SyndromesMucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 ProteinNeoplasm ProteinsNF-kappa BReceptors, Antigen, B-CellSignal TransductionAdaptor Proteins, Signal TransducingB-Cell CLL-Lymphoma 10 ProteinBCL10 protein, humanCARD11 protein, humanCARD Signaling Adaptor ProteinsCaspasesGuanylate CyclaseMALT1 protein, humanMucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 ProteinNeoplasm ProteinsNF-kappa BReceptors, Antigen, B-CellCARD11-BCL10-MALT1 signalosome complexCARMA1combined immunodeficiencycongenital B-cell lymphocytosisnext-generation sequencingnuclear factor κBparacaspaseprimary immunodeficiency diseases

Identifiers

PMID25087226
PMCPMC4167767
OpenAlexW1996305237

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.